Scleroderma is a connective tissue disease. Collagen is the strong fibrous stuff found in connective tissue. Connective tissue connects and supports parts of the body and is dense with blood vessels. While Scleroderma means "hard skin" this same hardening can happen to an organ or tissue because of rapid growth of too much collagen, which is my case. Most often death with Scleroderma is caused by pulmonary disease. About 150,000 to 300.000 in the U.S. have Scleroderma in the U.S., striking 4 times as many women as men. The cause of Scleroderma is unknown and there is not presently a cure. Scleroderma can lead to PH because it can cause scar tissue to build up in the lungs or heart. Roughly a third of systemic Scleroderma patients may develop mild PH. When mild, it can often go undetected for a number of years, not progressing. When symptoms are prevalent such as increased shortness of breath, it is much easier to diagnose and also much harder to treat. It is not known how often mild PH grows into serious PH as mine has. My pulmonary function tests have shown I have had mild PH in my lungs for several years. If a person has a diffusing capacity of less than 55 percent predicted, they probably have PAH. This is the ability of oxygen to pass from the air sacs of the lungs into the blood.
If a patient does not have a severe form of PH, doctors usually begin treatment by lowering lung pressures with calcium channel blockers, (Norvasc, Procardia, etc.), that come in pill form and are much lest expensive than alternative treatments. Unfortunately, few patients respond to this treatment.
If calcium channel blockers don’t work or a patient is in Class III, then endothelin receptor antagonists (traclear, etc.), some form of prostanoid theraphy (Flolan, Iloprost, ect.), and/or phosphodiesterase inhibitors (Viagra, etc,) will be considered. Letairis, which is what I just started is an endothelin receptor antagonist. Endothelin is present in the blood. Patients with PH triggered by other diseases tend to have higher-than-normal levels of it. It binds to receptors in the layer of smooth muscle cells that surround it. Once it binds. it causes vasoconstriction and retards the growth and development of new smooth muscle cells. It may also increase fibrous tissue formation. Designer molecules made in labs can keep endothelin from binding to receptors and are called endothelin receptor antagonists. Their great advantage is they come in pill form.
Before Flolan was available in 1995, class I or II PH patients lived an median of 6 years . Class III patients (of which I am one) lived 2.5 years and class IV patients lived only 6 months. Scleroderma patients with PH do not appear to live as long even with Flolan. When Scleroderma patients die it is usually from pulmonary disease, particularly pulmonary vascular disease. Letaris has shown that class III life expectancy can be increased to 5 years. I think this is with a patient whose PH is primary, not secondary such as me whose primary disease is Scleroderma.
My references regarding medical information come from the Pulmonary HYpertension Association, the Scleroderma Foundation, The Scleroderma Research Foundation, and The International Scleroderma Network. I am not a doctor, just a patient. Scleroderma affects each patient differently. What works for one patient may not work for another and may even be dangerous. I make an effort to provide reliable information. However, it is up to you to verify all facts and consult your own doctor before starting or discontinuing any treatments.
Friday, October 31, 2008
Wednesday, October 29, 2008
What is Pulmonary Hypertension?
I thought I would explain what is pulmonary hypertension. When pulmonary hypertension is diagnosed with no known cause, it is called primary pulmonary hypertension. When the cause is known, such as with me, the cause is Scleroderma, then it is known as secondary pulmonary hypertension. It is rare and without treatment it is usually a rapid fatal disease.
Blood enters the right side of your heart carrying very little oxygen. The right side of your heart pumps the blood into the arteries of your lungs. (Here is a fact: this is the only place in your body where your blood is blue). These arteries grow smaller and become arterioles, about the size of a hair. They are so small, the red blood cells can only go through one or two at a time. They then go through even tinier capillaries that surround the air sacs in your lungs. Here they take on oxygen from the lungs, return to the left side of the heart and are pumped out to the rest of your body.
PH usually starts in the lungs. My lungs are scarred from the Scleroderma and they don’t have the elasticity your lungs have nor the ability to move the oxygenated blood through the cells due to narrowing and scarring (fibrosis). My arteries and arterioles are stiff and scarred as well restricting blood flow even more. So I had high blood pressure in my lungs first as my lungs worked harder. When the lungs can’t work well, the pressure backs up into the heart which works harder to compensate for the lungs to push blood through. Eventually, your heart can’t keep up and there is less blood circulating in the lungs to pick up oxygen and you become tired, dizzy, and short of breath. Fainting, edema, chest pain, and dry cough may be present. You may feel like you are choking or hungry for air. I can feel my heart beat, clear into my eyeballs. It will thump, flutter and do other strange things. I have had fainting problems for several years. I get up to do something and I can feel the blackness closing in. I have to lay down for a while then get up and try again. It usually goes away. My oxygen tank has helped a lot.
Not much is known about PH, even less is known about PH with Scleroderma. It is a disease of the blood vessels of the lungs and heart. Tomorrow I will talk about the little bit that is known about Scleroderma and PH.
Blood enters the right side of your heart carrying very little oxygen. The right side of your heart pumps the blood into the arteries of your lungs. (Here is a fact: this is the only place in your body where your blood is blue). These arteries grow smaller and become arterioles, about the size of a hair. They are so small, the red blood cells can only go through one or two at a time. They then go through even tinier capillaries that surround the air sacs in your lungs. Here they take on oxygen from the lungs, return to the left side of the heart and are pumped out to the rest of your body.
PH usually starts in the lungs. My lungs are scarred from the Scleroderma and they don’t have the elasticity your lungs have nor the ability to move the oxygenated blood through the cells due to narrowing and scarring (fibrosis). My arteries and arterioles are stiff and scarred as well restricting blood flow even more. So I had high blood pressure in my lungs first as my lungs worked harder. When the lungs can’t work well, the pressure backs up into the heart which works harder to compensate for the lungs to push blood through. Eventually, your heart can’t keep up and there is less blood circulating in the lungs to pick up oxygen and you become tired, dizzy, and short of breath. Fainting, edema, chest pain, and dry cough may be present. You may feel like you are choking or hungry for air. I can feel my heart beat, clear into my eyeballs. It will thump, flutter and do other strange things. I have had fainting problems for several years. I get up to do something and I can feel the blackness closing in. I have to lay down for a while then get up and try again. It usually goes away. My oxygen tank has helped a lot.
Not much is known about PH, even less is known about PH with Scleroderma. It is a disease of the blood vessels of the lungs and heart. Tomorrow I will talk about the little bit that is known about Scleroderma and PH.
TV
I love Targets new TV ad showing such things as the new movie night which is staying home and watching a DVD. It shows the new coffee spot which is at a bistro table in your own kitchen. With the drastic changes in the economy the past few weeks, we are all having to make changes.
On the news tonight was a segment about people giving up important medications in order to have money for food. I can identify with that. Even though we have Kaiser (Brian will have it again in January), prescription drugs are a major part of out monthly cost of living. Yes, I did get a grant for Letairis, and Brian will hopefully get his next two months of aricept free from the drug company. There are other drugs we take to help our disease processes. Each month I have to decide which ones I feel I can get by without and which ones I really need. Month before last, I went without prednisone. My doctor freaked when I told him because I guess you can’t quit cold turkey like that. This month I went without my thyroid and my chloresterol meds. Those of you who heard me talk this month probably noticed how gravelly my voice was. This happens when I go without my thyroid med.
The news said that pharmacys are noticing a major change. There are a lot more prescriptions for anti-depressants and a lot of people are not refilling some essential drugs that could mean the difference between life and death, such as high blood pressure pills and anti-seizure medications. The news offered 3 solutions: 1) see if your drug manufacturer offers help in defraying the costs of your meds. 2) See if there are generic drugs available that offer the same benefits as a brand name drug. 3) If you have to cut back on your meds, talk to your doctors about which ones are the most necessary and which, if any are not. What ever you do, do not quit your meds cold turkey for even a short time as I did my prednisone since that is dangerous.
I think the next year will be a series of financial ups and downs. I think we all will rethink our spending habits as the Target ad is already showing. Talk about being in tune with today’s consumer! I commend Target for their insight. We have to decide what is important to living a full-filling life and what is not. I think we are going to find that things we couldn’t live without before are not that necessary now. My hope is that we become happier people without the stress of keeping up with the Joneses. By cutting back on spending hopefully we can cut back on our work hours and take time to enjoy our families and friends and to just smell the roses.
On the news tonight was a segment about people giving up important medications in order to have money for food. I can identify with that. Even though we have Kaiser (Brian will have it again in January), prescription drugs are a major part of out monthly cost of living. Yes, I did get a grant for Letairis, and Brian will hopefully get his next two months of aricept free from the drug company. There are other drugs we take to help our disease processes. Each month I have to decide which ones I feel I can get by without and which ones I really need. Month before last, I went without prednisone. My doctor freaked when I told him because I guess you can’t quit cold turkey like that. This month I went without my thyroid and my chloresterol meds. Those of you who heard me talk this month probably noticed how gravelly my voice was. This happens when I go without my thyroid med.
The news said that pharmacys are noticing a major change. There are a lot more prescriptions for anti-depressants and a lot of people are not refilling some essential drugs that could mean the difference between life and death, such as high blood pressure pills and anti-seizure medications. The news offered 3 solutions: 1) see if your drug manufacturer offers help in defraying the costs of your meds. 2) See if there are generic drugs available that offer the same benefits as a brand name drug. 3) If you have to cut back on your meds, talk to your doctors about which ones are the most necessary and which, if any are not. What ever you do, do not quit your meds cold turkey for even a short time as I did my prednisone since that is dangerous.
I think the next year will be a series of financial ups and downs. I think we all will rethink our spending habits as the Target ad is already showing. Talk about being in tune with today’s consumer! I commend Target for their insight. We have to decide what is important to living a full-filling life and what is not. I think we are going to find that things we couldn’t live without before are not that necessary now. My hope is that we become happier people without the stress of keeping up with the Joneses. By cutting back on spending hopefully we can cut back on our work hours and take time to enjoy our families and friends and to just smell the roses.
Monday, October 27, 2008
Marriage
I mislead you yesterday when I said my pills were $33 each. That would be true if I had a 3 month supply. I only have a 30 day supply so the cost is $110 per pill. I thank God each time I take one.
There is something to be said for 37 years of marriage. Brian and I were very young when we got married. It wasn’t until a year or two after we were married that our friends started living together without benefit of marriage. That never occurred to us. We both lived at home and then we were married. Now we have been married almost twice as long as we were single. We also waited five years before we had our kids which I think gave us a much more stable marriage. We have had a lot of fun along the way, playing some great pranks on each other and when we had Wendy and Kevin, they were not exempt. I think one of the secrets to our marriage is that we have made it fun. We have both grown as individuals, pursuing things that interested each of us separately. While we are secure in the knowledge that we are not dependent on each other to feel validated, we have found that our lives are much happier doing things together as well. As time went on, we found we were doing more things together than not. It of course helps that while Brian has a temper he has never used it against me. I didn’t even know he had a temper until after we were married. And I very seldom get angry so we compliment each other. We also respect each other. We have never said anything to each other that might hurt the other’s feelings. I, myself have never let those thoughts enter my mind. I think if you look for faults you are going to find them. Being a Pollyana, (put a long blond wig on me and Haley Mill’s cute face instead of my prednisone chipmunk cheeks, I would look just like her), I have always tried to find something good in everything and everyone. So always seeing the good in Brian is a natural for me. We have always said please and thank you to each other for everything, no matter how small. I am a firm believer that family comes first. Too many people treat others nicely because they want people to like them, then treat their family pretty crappy.
Now, even with his Alzheimer’s, Brian still tells me thank you each time I fix him a meal. He always tells me it is the best meal he has ever had, even if it is just soup and a sandwich. Sometimes Alzheimer’s patients personalities change and they lash out at loved ones. If anything, Brian is even nicer and more grateful for everything I do for him. And I am so happy that I can still do the essential things he needs.
This past year, we have both had some embarrassing moments but after 37 years of marriage, they are not embarrassing to us. We just help each other put things right and then laugh about it. I am sure that on this journey of ours we will have many, many more such moments and I hope that we will always be able to laugh.
Brian is having a harder time getting his thoughts across. But after 37 years of marriage, I can pretty much finish his sentences for him. I think this really keeps his frustration level much lower than what some people experience when they cannot get their thoughts across to others.
My hands don’t work right any more. Oh, I don’t have the stiffening and crippling in my hands that most scleroderma patients have. But I have the dropsies. And unlike Phebe on the tv show Friends, I am not very bendy. I can’t bend over to put my shoes on so Brian often has to try and help me. And several times a day I have to call Brian in to help me in my craft room and pick up my messes. We laugh and tell everyone that I am the brains and he is the brawn. Again, we complement each other.
I am sometimes baffled by the fact that we are both hampered by such harsh diseases. We have learned great coping skills throughout our marriage that so far have enabled us to enjoy each day and appreciate our time together.
There is something to be said for 37 years of marriage. Brian and I were very young when we got married. It wasn’t until a year or two after we were married that our friends started living together without benefit of marriage. That never occurred to us. We both lived at home and then we were married. Now we have been married almost twice as long as we were single. We also waited five years before we had our kids which I think gave us a much more stable marriage. We have had a lot of fun along the way, playing some great pranks on each other and when we had Wendy and Kevin, they were not exempt. I think one of the secrets to our marriage is that we have made it fun. We have both grown as individuals, pursuing things that interested each of us separately. While we are secure in the knowledge that we are not dependent on each other to feel validated, we have found that our lives are much happier doing things together as well. As time went on, we found we were doing more things together than not. It of course helps that while Brian has a temper he has never used it against me. I didn’t even know he had a temper until after we were married. And I very seldom get angry so we compliment each other. We also respect each other. We have never said anything to each other that might hurt the other’s feelings. I, myself have never let those thoughts enter my mind. I think if you look for faults you are going to find them. Being a Pollyana, (put a long blond wig on me and Haley Mill’s cute face instead of my prednisone chipmunk cheeks, I would look just like her), I have always tried to find something good in everything and everyone. So always seeing the good in Brian is a natural for me. We have always said please and thank you to each other for everything, no matter how small. I am a firm believer that family comes first. Too many people treat others nicely because they want people to like them, then treat their family pretty crappy.
Now, even with his Alzheimer’s, Brian still tells me thank you each time I fix him a meal. He always tells me it is the best meal he has ever had, even if it is just soup and a sandwich. Sometimes Alzheimer’s patients personalities change and they lash out at loved ones. If anything, Brian is even nicer and more grateful for everything I do for him. And I am so happy that I can still do the essential things he needs.
This past year, we have both had some embarrassing moments but after 37 years of marriage, they are not embarrassing to us. We just help each other put things right and then laugh about it. I am sure that on this journey of ours we will have many, many more such moments and I hope that we will always be able to laugh.
Brian is having a harder time getting his thoughts across. But after 37 years of marriage, I can pretty much finish his sentences for him. I think this really keeps his frustration level much lower than what some people experience when they cannot get their thoughts across to others.
My hands don’t work right any more. Oh, I don’t have the stiffening and crippling in my hands that most scleroderma patients have. But I have the dropsies. And unlike Phebe on the tv show Friends, I am not very bendy. I can’t bend over to put my shoes on so Brian often has to try and help me. And several times a day I have to call Brian in to help me in my craft room and pick up my messes. We laugh and tell everyone that I am the brains and he is the brawn. Again, we complement each other.
I am sometimes baffled by the fact that we are both hampered by such harsh diseases. We have learned great coping skills throughout our marriage that so far have enabled us to enjoy each day and appreciate our time together.
Sunday, October 26, 2008
This N That
I took my first $33 pill Friday night. Most nights I do not sleep well, but I slept well Friday night. Sat. morning, Brian and I got up and got ready to go to our grandson’s birthday party. We picked up Kevin around 12:45 and had a wonderful time at Joshua’s 7th birthday party. He is so sweet and loving. Our grandkids, Joshua and Melissa, are such a joy in our lives.
We took Kevin to work at 4 p.m. and went home. I was exhausted when we got home at 4:30 so after I helped Brian get settled I laid down to read and maybe take a nap. I ended up going right to sleep and other than helping Brian a couple of times, I slept the night away, getting up at 10 a.m. this morning. I thought maybe the Letairis was at fault for my extreme fatigue but I am much better today. I haven’t had any other effects of Letairis (knock on wood).
Fatigue is a major concern for me. I tried to explain to one of my doctors once that some days I am so tired, it is too hard to hold my head on my shoulders. Sleep is another problem. I have always been a night owl. Since Brian has been off work he has gradually come over to the dark side too. I try to go to bed by 2 a.m. or else I get Brian and Belle, our cat, telling me it is time for bed. Brian used to go right to sleep and I take a vicodin and read until my body settles down enough to let me sleep, usually after a couple of hours. Sometimes I think I am ready to sleep, turn off my bedside lamp and immediately I am wide awake again. Some days I don’t get to sleep until 9 a.m. or later. Then I will sleep for 10 or 12 hours at a time.
For about the last week or more, Brian has been having trouble falling asleep. He gets in bed, then he will get out of bed, then in and out and in and out. He kind of wanders a bit but doesn’t know what he wants or needs. After a bit he will finally settle down and sleep. Some Alzheimer’s patients have a tendency to take off and wander outside of their home, getting lost in the process. I have always thought Brian would not be a wanderer but now I wonder if Brian’s behavior now is indicative of a wandering soul in the future. It isn’t just when he goes to bed that he is up and down, he doesn’t seem to be able to sit still for long either and is up and down and walking around a lot in the daytime too. If this is all I have to complain about today, then life is good!
We took Kevin to work at 4 p.m. and went home. I was exhausted when we got home at 4:30 so after I helped Brian get settled I laid down to read and maybe take a nap. I ended up going right to sleep and other than helping Brian a couple of times, I slept the night away, getting up at 10 a.m. this morning. I thought maybe the Letairis was at fault for my extreme fatigue but I am much better today. I haven’t had any other effects of Letairis (knock on wood).
Fatigue is a major concern for me. I tried to explain to one of my doctors once that some days I am so tired, it is too hard to hold my head on my shoulders. Sleep is another problem. I have always been a night owl. Since Brian has been off work he has gradually come over to the dark side too. I try to go to bed by 2 a.m. or else I get Brian and Belle, our cat, telling me it is time for bed. Brian used to go right to sleep and I take a vicodin and read until my body settles down enough to let me sleep, usually after a couple of hours. Sometimes I think I am ready to sleep, turn off my bedside lamp and immediately I am wide awake again. Some days I don’t get to sleep until 9 a.m. or later. Then I will sleep for 10 or 12 hours at a time.
For about the last week or more, Brian has been having trouble falling asleep. He gets in bed, then he will get out of bed, then in and out and in and out. He kind of wanders a bit but doesn’t know what he wants or needs. After a bit he will finally settle down and sleep. Some Alzheimer’s patients have a tendency to take off and wander outside of their home, getting lost in the process. I have always thought Brian would not be a wanderer but now I wonder if Brian’s behavior now is indicative of a wandering soul in the future. It isn’t just when he goes to bed that he is up and down, he doesn’t seem to be able to sit still for long either and is up and down and walking around a lot in the daytime too. If this is all I have to complain about today, then life is good!
Friday, October 24, 2008
Grant
Great news! I was awarded the grant for Letairis! I received my first 30 day supply by Fed Ex today from Accreedo, the pharmacy that handles the special drugs for PAH patients (pulmonary arterial hypertension). That was so fast! I only sent my form back by mail to my doctor the beginning of last week. I will take my first 5mg pill tonight. It will take several months to see if this drug is working. There are 10mg doses as well if this dosage doesn’t do what we hope. The side effects are: lowering of red blood cell counts, swelling from fluid retention, stuffy nose and inflamed nasal passages, hot flashes, heart palpatations, red and sore throat, stomach pain, constipation, shortness of breath (your patient chart shows sob, so don’t take it personally), and headache. Several of these side effects I already have due to other causes, so I think I can handle these just fine. Some of the drugs I have taken in the past have had to be weighed as to whether the benefits out weigh the side effects. Prednisone is such a drug when taken long term as I do. Letaris doesn’t seem to have that problem. Every couple months I have to perform the 6 minute walk test. I am not sure how I am going to do that when I cannot walk more than a few feet partly because I cannot breathe and walk at the same time but also because of the avascular necrosis. Scleroderma has filled my body with scar tissue and that and prednisone have reduced the ability of my blood to get to my hips and legs. It is very painful to move and walk and weakens my bones. Without prednisone, the alternative is much worse. The 6 minute walk test is to see if the drug is working to slow down the disease process and help me breathe a little easier. I am sure my team of doctors and I will figure out something to test the effects of letaris. Hopefully I will eventually see improvement in my day to day living.
I have a wonderful team of doctors at Kaiser. They include a pulmonary specialist, rhuematologist, gastroenterologist, and a doctor who specializes in critical care. If I see one doctor, within one day all of them have the info from that visit and they regularly communicate with each other about my disease process. I see my gastro doc in a about 2 weeks. Because of my anemia, it is believed I have a slow bleed in my stomach so will probably have a endoscopy scheduled shortly thereafter. I haven’t any major digestive problems for a while so I am definitely due for an endo. For me, it is a great way to nap my day away.
I have always had wonderful care at Kaiser. I suggest if you do not like your doctor or the care you are receiving, find another doctor. They are out there and it can make a huge difference in how you view life.
I have a wonderful team of doctors at Kaiser. They include a pulmonary specialist, rhuematologist, gastroenterologist, and a doctor who specializes in critical care. If I see one doctor, within one day all of them have the info from that visit and they regularly communicate with each other about my disease process. I see my gastro doc in a about 2 weeks. Because of my anemia, it is believed I have a slow bleed in my stomach so will probably have a endoscopy scheduled shortly thereafter. I haven’t any major digestive problems for a while so I am definitely due for an endo. For me, it is a great way to nap my day away.
I have always had wonderful care at Kaiser. I suggest if you do not like your doctor or the care you are receiving, find another doctor. They are out there and it can make a huge difference in how you view life.
Thursday, October 23, 2008
Is This Enough?
Brian lost his Kaiser coverage last month and this month we had to pay full price for his aricept. It runs over $200 per month. He will get his Kaiser coverage back January first and will go back to paying $50 per month. This is a tier three drug, like an orphan drug but many more people need it so the price comes down. In the meantime, aricept does have a program that we qualify for if you have a certain income level and no drug coverage. They pay the costs of the aricept. Obviously, it is a great program. But isn’t it a shame that people on social security disability have to pay the high costs we pay for our medicines that are literally life saving?
The estimated cost of bringing a single drug through screening processes and FDA procedures to obtain approval is between $350 – 500 million. Companies spend nearly $21 billion just on research and development for treatments and it can take up to 10 years just to bring a single drug into the market. Prior to the Orphan Drug act, very few orphan drugs were brought to market.
In 1983, the Orphan Drug act was introduced to facilitate the development of orphan drugs and other medical products by offering incentives such as seven years of marketing exclusivity after approval of the orphan drug product and offering research grants to reduce the costs incurred in the development of orphan products. A rare disease is considered one that occurs in less than 200,000 individuals in the U.S. Since passage of the Orphan Drug Act, more than 300 drugs to treat rare disease have come to market to help an estimated 12 million Americans. This has been confirmed online by Janet Woodcock, director of FDA’s Center for Drug Evaluation and Research.
My question is, Is this enough? There are over 6000 recognized rare diseases. I am grateful they have Letairis for me (especially if it works) and Aricept for Brian. But my heart goes out to those parents who’s children are diagnosed with a rare disease and there is no drug to help them. I know that companies have to answer to their shareholders to make money and yes the Orphan Drug Act has helped. I can only quote statistics I have found. I don’t have the answers. I just feel so badly for those poor parents and their children who still need a miracle drug. I feel guilty to be so lucky that Brian and I have drugs to help us.
The estimated cost of bringing a single drug through screening processes and FDA procedures to obtain approval is between $350 – 500 million. Companies spend nearly $21 billion just on research and development for treatments and it can take up to 10 years just to bring a single drug into the market. Prior to the Orphan Drug act, very few orphan drugs were brought to market.
In 1983, the Orphan Drug act was introduced to facilitate the development of orphan drugs and other medical products by offering incentives such as seven years of marketing exclusivity after approval of the orphan drug product and offering research grants to reduce the costs incurred in the development of orphan products. A rare disease is considered one that occurs in less than 200,000 individuals in the U.S. Since passage of the Orphan Drug Act, more than 300 drugs to treat rare disease have come to market to help an estimated 12 million Americans. This has been confirmed online by Janet Woodcock, director of FDA’s Center for Drug Evaluation and Research.
My question is, Is this enough? There are over 6000 recognized rare diseases. I am grateful they have Letairis for me (especially if it works) and Aricept for Brian. But my heart goes out to those parents who’s children are diagnosed with a rare disease and there is no drug to help them. I know that companies have to answer to their shareholders to make money and yes the Orphan Drug Act has helped. I can only quote statistics I have found. I don’t have the answers. I just feel so badly for those poor parents and their children who still need a miracle drug. I feel guilty to be so lucky that Brian and I have drugs to help us.
Wednesday, October 22, 2008
Letairis
Yesterday I told you about the new drug Letairis. It is so expensive that my first co-payment is $3,377.80. No, I am not kidding...Because of my other drugs, I am in the donut hole of my RX coverage but I will reach my cap and my coverage turns to catastrophic and I only pay about $233 for Letairis per month after that until the first of the year when I have to reach my cap again. There are only 8 pharmacies that carry this drug and I was called by one of them on Monday to set up the program. I was refereed to a company called Caring Voice that helps people like me with their co-payments. They are located in Pennsylvania while I am located in California. Anyway, they are writing a grant that will probably cover most if not all of my costs for this drug. Imagine that, a grant that I don’t have to pay back from some folks who don’t even know me. Just when I was just happy that Kaiser will pay a big chunk of this drug that costs $35,000 to $45,000 per year. I should know in the next week if the grant goes through. Yes, George, it is a wonderful life!
Tuesday, October 21, 2008
Heart Catherization
So, I had a heart catherization procedure about two weeks ago. I wasn’t looking forward to it. Although I live with pain on a daily basis, I am against adding to my pain no matter what the procedure. But my doctors have been after me for the past year to have this procedure so I finally agreed. And you know what? It wasn’t bad at all. In fact, I found it much more ticklish than painful and got laughing so hard I couldn’t keep still. The doctor and nurses couldn’t believe how ticklish I was and said most people just lay there. They never had anyone giggle let alone out and out laugh. They give you something to take the edge off and use a local anesthetic. I think most people go to sleep but I struggled against it and stayed awake. How often do you get to see your own heart, let alone watch a wire in it. I thought it fascinating. The results were not good. In addition to my pulmonary fibrosis (lung scaring from scleroderma, I have pulmonary hypertension, high blood pressure in my heart. Had this happened five years ago, there would have been no good treatments available. But last June, 2007, a drug called Letairis was approved for this rare disease. It has been found to give as much as an additional 2 and a half years to one’s life. And you can see what a difference a few years can make. The downside is that this is considered an orphan drug, and is very expensive. But you do what you have to to buy time in this wonderful world. And today I found out there are more wonderful people out there that I will tell you about tomorrow.
Monday, October 20, 2008
Motorized Wheelchairs
I love my motorized wheelchair. With my avascular necrosis, somedays it hurts so badly to move, let alone walk. And to breathe and walk at the same time...well that is a laugh. To breathe and laugh at the same time...well....anyway, my motorized wheelchair is so great around the house. And it is so very comfortable, too. But what kind of company makes a motorized wheelchair that doesn't fit in a normal car. Yes, while my wheelchair is great at home, I can not take it with me. I have a regular wheelchair that will fit in my trunk, but it is bulky and heavy. And it needs to be pushed which Brian can not do any more. Oh, he can push ok, but he can not maneuver it or understand my directions. My motorized wheelchair would be so wonderful out in the real world. The makers don't come out and tell you that it won't fit in a normal car, you have to find out the hard way. My doctor ordered it, A Kaiser healthcare professional came out to my home, interviewed me, confirmed that I needed one and ordered it. The healthcare professional told me to let my landlord know I needed a ramp for it since every other apartment building has wheelchair ramp access except mine. I guess by law the apartment building has to have so many spaces for so many apartments. My apartment manager showed me an article in an apartment rental magazine that said apartment owners did not have to build ramps for their tenants. She said that to put in a handicap ramp would run $3,500 and that I could do it if I wanted to, but the money would have to go into an escrow account first. Now, I am sorry, Miss Landlady, I do not have $3,500 but you do have about $2000 of our money on deposit. Seems like we could reach a compromise, but no can do....so even if I could get the chair into my car, I don't have a ramp. If I could figure out how to get the chair into my car, I could buy a portable ramp that I could use if space was available. Anyway, I digress....watch the ads for those motorized chairs. They state how wonderful they are for maneuverability in your home. And I tell you, it is so wonderful, it really is...but no where does it state it doesn't work in a car. Easy, you say....Get another car. Yes, I could but this car will be paid for sooner rather than later and another car just means a new loan. I am able to drive now, but with the avascular necrosis in my hips, that may not be a possibility down the line. So, while having the chair with me when I go out would be nice, I can get by without it for now so I just have taken a wait and see attitude. I guess if it works, don't try to fix it.
Motorized Wheelchairs
I love my motorized wheelchair. With my avascular necrosis, somedays it hurts so badly to move, let alone walk. And to breathe and walk at the same time...well that is a laugh. To breathe and laugh at the same time...well....anyway, my motorized wheelchair is so great around the house. And it is so very comfortable, too. But what kind of company makes a motorized wheelchair that doesn't fit in a normal car. Yes, while my wheelchair is great at home, I can not take it with me. I have a regular wheelchair that will fit in my trunk, but it is bulky and heavy. And it needs to be pushed which Brian can not do any more. Oh, he can push ok, but he can not maneuver it or understand my directions. My motorized wheelchair would be so wonderful out in the real world. The makers don't come out and tell you that it won't fit in a normal car, you have to find out the hard way. My doctor ordered it, A Kaiser healthcare professional came out to my home, interviewed me, confirmed that I needed one and ordered it. The healthcare professional told me to let my landlord know I needed a ramp for it since every other apartment building has wheelchair ramp access except mine. I guess by law the apartment building has to have so many spaces for so many apartments. My apartment manager showed me an article in an apartment rental magazine that said apartment owners did not have to build ramps for their tenants. She said that to put in a handicap ramp would run $3,500 and that I could do it if I wanted to, but the money would have to go into an escrow account first. Now, I am sorry, Miss Landlady, I do not have $3,500 but you do have about $2000 of our money on deposit. Seems like we could reach a compromise, but no can do....so even if I could get the chair into my car, I don't have a ramp. If I could figure out how to get the chair into my car, I could buy a portable ramp that I could use if space was available. Anyway, I digress....watch the ads for those motorized chairs. They state how wonderful they are for maneuverability in your home. And I tell you, it is so wonderful, it really is...but no where does it state it doesn't work in a car. Easy, you say....Get another car. Yes, I could but this car will be paid for sooner rather than later and another car just means a new loan. I am able to drive now, but with the avascular necrosis in my hips, that may not be a possibility down the line. So, while having the chair with me when I go out would be nice, I can get by without it for now so I just have taken a wait and see attitude. I guess if it works, don't try to fix it.
Friends
Sunday, October 19, 2008
Friends
It just amazes me that we have such amazing friends. Not that they are amazing but that we have so many. Brian and I do not go out much. (Some days we work at the art of doing nothing. Won't be long before we have that perfected!) If we do, it is usually with Wendy or Kevin, our kids, or Kim and Wayne, our best friends who just happen to be Brian's sister and her husband. And they are so wonderful about helping us if we need it. Getting us drinks so that we don't have to get up off our butts. Helping Brian find the bathroom maze that he faces whenever we go out. That is so nice, but they are family and they are more in tune with what we are going through. But our friends do the same things too. Last night at our Disney meeting, if we needed anything, someone was there who was better able to get it for us. If we needed trash thrown away, it was gone. It humbles me when people help us. I had to learn to accept it because, frankly, it sure makes living easier. But our friends do it without thinking twice about it. And that is what amazes me. The capacity of the human heart to care and to love unconditionally. That is one of our greatest reasons to go on battling our disease processes. To all of our friends and relatives...thank you. You are what makes us want to keep going each day. Thank you!
Friends
It just amazes me that we have such amazing friends. Not that they are amazing but that we have so many. Brian and I do not go out much. (Some days we work at the art of doing nothing. Won't be long before we have that perfected!) If we do, it is usually with Wendy or Kevin, our kids, or Kim and Wayne, our best friends who just happen to be Brian's sister and her husband. And they are so wonderful about helping us if we need it. Getting us drinks so that we don't have to get up off our butts. Helping Brian find the bathroom maze that he faces whenever we go out. That is so nice, but they are family and they are more in tune with what we are going through. But our friends do the same things too. Last night at our Disney meeting, if we needed anything, someone was there who was better able to get it for us. If we needed trash thrown away, it was gone. It humbles me when people help us. I had to learn to accept it because, frankly, it sure makes living easier. But our friends do it without thinking twice about it. And that is what amazes me. The capacity of the human heart to care and to love unconditionally. That is one of our greatest reasons to go on battling our disease processes. To all of our friends and relatives...thank you. You are what makes us want to keep going each day. Thank you!
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